BASICS

DESCRIPTION

Papilledema is bilateral optic nerve swelling from elevated intracranial pressure (ICP).

EPIDEMIOLOGY

Incidence

Related to incidence of underlying cause of raised ICP. In young infants with open sutures, papilledema may not occur.

Prevalence

Unknown

RISK FACTORS

• Hydrocephalus with or without shunting

• Head trauma

• Premature infants with grade 3 or 4 intraventricular hemorrhage

• For pseudotumor cerebri: certain medications (in particular systemic retinoids, steroid withdrawal, growth hormone, tetracycline) and systemic disorders (e.g., Down syndrome, obesity)

• Craniosynostosis syndromes

• Brain tumor

Genetics

No known genetic predisposition

Pediatric Considerations

• Examination may be difficult, so it is imperative to identify those children who may be at risk of having papilledema.

• Papilledema may be absent even in the presence of markedly raised ICP in infants with open sutures or in shaken baby syndrome.

GENERAL PREVENTION

Early recognition and treatment of increased ICP

PATHOPHYSIOLOGY

• Increased ICP is transmitted to the optic nerve causing reduced axoplasmic flow initially causing papilledema and ultimately leading to optic atrophy.

– Brain tumors, especially infratentorial tumors, cause obstruction of normal intraventricular flow of cerebral spinal fluid (obstructive hydrocephalus).

– Idiopathic intracranial hypertension (IIH) (pseudotumor cerebri), either primary or secondary, leading to decreased absorption of CSF and subsequent ICP elevation

ETIOLOGY

• Elevated ICP from any cause

• IIH can be either primary or secondary.

COMMONLY ASSOCIATED CONDITIONS

See Risk Factors

DIAGNOSIS

HISTORY

• Headache; especially those associated with awakening from sleep or awakening in the morning. Headache in a patient with a known cause for high ICP such as hydrocephalus or a teenager taking retinoids for acne is an ominous sign.

– Transient vision blurring (visual obscuration) lasting for a few seconds occurring several times per day

– Nausea and vomiting

– Infants and preverbal children may present with listlessness, irritability, and somnolence

PHYSICAL EXAM

• Normal visual acuity early. Pupils usually normal. Cranial nerves can be affected, especially sixth nerve palsy. When visual acuity is affected, it can be lost quickly. Later or with recurrences (as in shunt malfunctions) vision can be further reduced. Color vision may be affected before visual acuity falls.

• Swollen optic nerve with engorged veins on the optic nerve surface

• Nerve fiber layer edema blurring the disc margin and covering blood vessels

• Peripapillary and disc hemorrhages

• Loss of spontaneous venous pulsations. Venous pulsations are an important observation. When present they indicate normal ICP. When absent they are of no value unless they were present on a prior examination.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• Lumbar puncture for opening pressure, cells, and protein. Normal opening pressure less than 180 mm Hg CBC count might detect anemia or leukemia.

– Urinalysis for diabetes insipidus

Imaging

Initial approach

• MRI (preferred) or computerized axial tomography (CAT) scan to rule out intracranial mass and diagnose enlarged ventricles or small ventricles in cerebral edema/pseudotumor

• Optic nerve ultrasound can document swollen nerve sheath and help in diagnosing drusen of the optic nerve head (main cause of pseudopapilledema).

Follow-up & special considerations

• Papilledema may take 4–6 weeks to resolve after ICP normalized.

• Optic atrophy can occur in long term. Follow patients for visual acuity, afferent pupillary defect, and color vision.

Diagnostic Procedures/Other

Visual field may show early defects near or adjacent to the blind spot or enlarged blind spot.

DIFFERENTIAL DIAGNOSIS

• Optic nerve head drusen

• Hyperopic disc

• Optic neuritis in children presents with asymmetric and/or severe vision loss and asymmetric optic nerve swelling. The vision loss is a key difference in presentation of papilledema versus optic neuritis.

• Papillitis (Epstein–Barr virus [EBV] infection, vasculitis, uveitis). Associated with vision loss.

TREATMENT

Determined mostly by the cause of the papilledema. No specific treatment of optic nerve involvement

MEDICATION

• Ventriculoperitoneal shunt for obstructive hydrocephalus or lumboperitoneal shunt is used primarily in IIH.

– Optic nerve sheath fenestration is used as first line at many centers for IIH.

• Medications also may be used as first-line treatment in IIH. Acetazolamide (Diamox) (10–15 mg/kg divided q.i.d.) and/or prednisone 1 mg/kg and/or diuretic (e.g., furosemide)

– Repeat lumbar punctures to remove a volume of CSF. Useful in IIH both primary and secondary. Can be curative.

– Cessation of medication causing IIH might be the primary treatment, for example, in a teenager being treated with retinoids for acne.

ADDITIONAL TREATMENT

Issues for Referral

Headaches with questionable optic nerve exam, especially those accompanied by visual obscuration or reduction in visual acuity, should be seen by ophthalmologist.

IN-PATIENT CONSIDERATIONS

Initial Stabilization

Hospitalization is done as needed by neurosurgery, neurology, or neuro-oncology.

Discharge Criteria

• Follow-up with ophthalmology usually within a few weeks following surgical procedure with shunting or tumor treatment, sooner if vision loss has already occurred.

– Follow-up sooner and ongoing if cerebral edema is being treated with systemic steroids and tapering or withdrawal is initiated.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patients with shunts should have eye exams periodically to document stability of optic nerve appearance and function.

Patient Monitoring

• Visual fields are useful to monitor stability of the optic nerve. Good computerized visual fields are difficult until the child is 8 or 9 years old.

– Ophthalmic exam should be done periodically. The follow-up ophthalmic exam (along with the visual field) is a front line defense for detecting occult shunt malfunction or early recurrent asymptomatic increased ICP.

DIET

• For IIH in obese children dietary restriction is indicated.

• Vitamin A toxicity may cause IIH.

PATIENT EDUCATION

• Recognizing signs of shunt malfunction

– Recognizing the need for routine ophthalmology follow-up in patients with shunts including need for yearly visual fields and funduscopic evaluation of optic nerve.

PROGNOSIS

• With normalization of ICP, the prognosis for vision is good.

– Recurrence of elevated pressure with shunt malfunction could cause vision loss from optic atrophy.

COMPLICATIONS

Recurrence as a result of shunt malfunction or recurrence of any underlying cause of increased ICP is an uncontrolled cause of IIH.

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